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| 1 | Expanding etiology of progressive familial intrahepatic cholestasis显示文摘BACKGROUND Progressive familial intrahepatic cholestasis(PFIC)refers to a disparate group of autosomal recessive disorders that are linked by the inability to appropriately form and excrete bile from hepatocytes,resulting in a hepatocellular form of cholestasis.While the diagnosis of such disorders had historically been based on pattern recognition of unremitting cholestasis without other identified molecular or anatomic cause,recent scientific advancements have uncovered multiple specific responsible proteins.The variety of identified defects has resulted in an ever-broadening phenotypic spectrum,ranging from traditional benign recurrent jaundice to progressive cholestasis and end-stage liver disease.AIM To review current data on defects in bile acid homeostasis,explore the expanding knowledge base of genetic based diseases in this field,and report disease characteristics and management.METHODS We conducted a systemic review according to PRISMA guidelines.We performed a Medline/PubMed search in February-March 2019 for relevant articles relating to the understanding,diagnosis,and management of bile acid homeostasis with a focus on the family of diseases collectively known as PFIC.English only articles were accessed in full.The manual search included references of retrieved articles.We extracted data on disease characteristics,associations with other diseases,and treatment.Data was summarized and presented in text,figure,and table format.RESULTS Genetic-based liver disease resulting in the inability to properly form and secrete bile constitute an important cause of morbidity and mortality in children and increasingly in adults.A growing number of PFIC have been described based on an expanded understanding of biliary transport mechanism defects and the development of a common phenotype.CONCLUSION We present a summary of current advances made in a number of areas relevant to both the classically described FIC1(ATP8B1),BSEP(ABCB11),and MDR3(ABCB4)transporter deficiencies,as well as more recently described gene mutations--TJP2(TJP2),FXR(NR1H4),MYO5B(MYO5B),and others which expand the etiology and understanding of PFIC-related cholestatic diseases and bile transport. | Sarah AF Henkel Judy H Squires Mary Ayers Armando Ganoza Patrick Mckiernan James E Squires | 2019 | World Journal of Hepatology2019，11，5： | 13 |
| 2 | 镁合金挤压成形过程中的成形载荷分析(英文)显示文摘采用数值模拟方法研究挤压成形过程中工艺参数对AZ31镁合金挤压载荷的影响,选取挤压温度、挤出比、摩擦因数和冲压速度作为主要参数。采用有限元法(FEM)和人工神经网络(ANN)方法对试验结果进行分析,建立可预测成形载荷的数值模型。将实验结果和数值分析结果进行比较,结果表明,对于所有给定的挤压比和冲压速度,在较低的挤压温度下,摩擦因数对挤压载荷的影响最大。此外,当挤压比较高时,需要较高的加工温度以便获得较低的挤压载荷。结果还表明,在所有的挤压比和挤压温度下,提高挤压速度会导致成形载荷显著增加。 | ?nder AYER | 2019 | Transactions of Nonferrous Metals Society of China2019，29，4： | 10 |
| 3 | 美国体育教学研究的新进展显示文摘20世纪80年代初,美国比较系统的关于中小学体育教学的研究开始起步,而体育教师教育专业的知识基础也处于早期发展阶段。从那时起到现在,美国中小学体育的性质和状况已发生了显著的变化。这种变化,是基于所进行的首批对中小学体育的教学研究,以及这之后多年的坚实的科学实证研究。由此,关于体育教学的知识已经取得了难以置信的发展。 | Suzan F. Ayers Lynn D. Housner 梁国立 | 2006 | 课程.教材.教法2006，26，6： | 8 |
| 4 | 终末期肾病患者大血管和微血管内皮功能障碍与亚临床心功能不全的关系显示文摘终末期肾病(end-stagerenaldisease,ESRD)患者的心脏衰竭和心血管病死亡率较高,而我们对此缺乏深入透彻的了解,是否存在可变因素导致了这些高危患者发生心功能不全。为了评估血管内皮功能能否作为ESRD患者潜在的心功能不全的可变因素,研究人员应用横断面研究在一个控制良好的ESRD队列中,分析大血管和微血管功能障碍与左、右心功能不全的相关性。 | 刘莉 叶鹏 Dubin RF Guajardo I Ayer A Mills C Donovan C Beussink L Scherzer R Ganz P Shah SJ | 2016 | 中华高血压杂志2016，24，9： | 5 |
| 5 | Surgical techniques used during caesarean section operations: results of a national survey of practice in the UK显示文摘 | Lucy Tully Simon Gates Peter Brocklehurst Kirstie McKenzie-McHarg Sarah Ayers | 2002 | European Journal of Obstetrics and Gynecology2002，，2： | 2 |
| 6 | Sulfate and methane- sulfonate in the ocean and the marine atmosphere: a global view显示文摘 | Ayers G P Ivey J P Goodman H S | 1986 | Science1986，221，： | 1 |
| 7 | Do firms purchase the pooling method? 显示文摘 | Ayers B C | 2002 | Review of Accounting Studies2002，7，1： | 1 |
| 8 | Preparation of nanocomposite materials containing WS2, δ-WN, Fe3O4, or Fe9S10 in a silica aerogel host 显示文摘 | Ayers M R Song X Y Hunt A J | 1996 | Journal of Materials Science1996，31，： | 1 |
| 9 | Coalbed gas systems,reservoirs,and production and a review of contrasting cases from the San Juan and Powder River basin显示文摘 | Ayers W B Jr | 2002 | AAPG Bulletin2002，86，11： | 1 |
| 10 | Sausage: protein threading with flexible force fields显示文摘 | Thomas Huber Anthony J Russell Daniel Ayers | 1999 | Bioinformatics1999，15，12： | 1 |
| 11 | Glyphmaker: creating customized visualization of complex data 显示文摘 | RIBARSKY W AYERS E EBLE J | 1995 | IEEE Computer1995，27，7： | 1 |
| 12 | Aptamer-based multiplexed pro- teomic technology for biomarker discovery显示文摘 | Gold L Ayers D Bertino J | 2010 | PLoS One2010，5，15： | 1 |
| 13 | Inhibition of interleukin -33 signaling attenuates the severity of experimental arthritis显示文摘 | Palmer G Talabot Ayer D Lamacchia C | 2009 | Arth Rheum2009，60，3： | 1 |
| 14 | Profile chan-ges associated with maxillary incisor retraction in the postad-olescent orthodontic patient显示文摘 | Kokodynski RA Marshall SD Ayer W | 1997 | Int J Adult Orthodon Or-thognath Surg1997，12，2： | 1 |
| 15 | A new grading system evaluating bleeding scale in filament perforation subarachnoid hemorrhage rat model显示文摘 | SUGAWARA T AYER R JADHAV V | 2008 | J Neurosci Methods2008，167，2： | 1 |
| 16 | Dysfunctional adiposity and the risk of prediabetes and type 2 diabetes in obese adults显示文摘 | Neeland IJ Turer AT Ayers CR | 2012 | JAMA2012，308，11： | 1 |
| 17 | Robotic positioning of standard electro- physiology catheters : a novel approach to catheter robotics 显示文摘 | Knight B Ayers GM Cohen TJ | 2008 | J lnva- sire Cardio12008，20，5： | 1 |
| 18 | Gadolinium is detectable within the tissue of patients with nephrogenic systemic fibrosis显示文摘 | High WA Ayers RA Chandler J | 2007 | J Am Acad Dermatol2007，56，： | 1 |
| 19 | A variable rate, direct nozzle injection field spray显示文摘 | Rockwell A D Ayers P D | 1996 | Applied Engineering in Agriculture1996，12，5： | 1 |
| 20 | Alkaloids of shepherdiaargentea and shepherdiacanadensis 显示文摘 | Ayer W A Browne L M | 1974 | Can J Chem1974，52，： | 1 |
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